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儿童霍奇金淋巴瘤的临床病理特征和治疗结果:来自沙特阿拉伯三级护理中心的报告。

Clinicopathologic features and therapy outcome in childhood Hodgkin's lymphoma: a report from tertiary care center in Saudi Arabia.

机构信息

Department of Pediatric Hematology/Oncology, Prince Sultan Military Medical City, Riyadh, Riyadh, 11159, Saudi Arabia.

Department of Central Military Laboratory and Blood Bank, Prince Sultan Military Medical City, Riyadh, Saudi Arabia.

出版信息

J Egypt Natl Canc Inst. 2021 Aug 16;33(1):21. doi: 10.1186/s43046-021-00078-0.

Abstract

BACKGROUND

Hodgkin lymphoma (HL) is lymphoid neoplasm usually affecting lymphatic system; it accounts 3.6% of cancers in Saudi Arabia. Modern treatment protocols had shown particular success rates in overall-survival (OS) and event-free-survival (EFS). In our study, we reviewed the medical records of 80 pediatric and young adolescent patients diagnosed HL from January 2006 to July 2020, treated at tertiary care hospital in Riyadh, Saudi Arabia. Demographic, clinical, and pathological data were explored. First line therapy was ABVD, COG, COPP, R-CHOP, or radiotherapy alone in 53/80 (66.4%), 24/80 (30%), 1/80 (1.2%), 1/80 (1.2%), or 1/80 (1.2%) patients; respectively. Response assessment was done by CT + / - PET scan after first 2 cycles then every 2 cycle and end of therapy. Another assessment was done if any clinical suspicion of recurrence.

RESULTS

Median age 11 (range 3-16) years. Males to females 1.3:1. Seventy-two out of eighty (90%) patients showed first complete remission (CR1) and maintained remission for median 40 (range 7-136) months. Eight out of eighty (10%) patients showed refractory disease. Nineteen patients received salvage therapy (ICE or ESHAP/brentuximab vedotin or gemcitabine/brentuximab vedotin), 14/19 (73.7%) had 2nd complete remission (CR2) for median time 24 (ranged 9-78) months, while 5/19 (26.3%) did not show any response. Five-year OS and EFS were 95% and 75%. Two patients had 2ry malignant neoplasms, one had AML and died, the other had malignant fibrous histocytoma and still alive. None of our patients had fertility problem. Also, they did not experience chronic pulmonary or cardiotoxicity. Classic Hodgkin's lymphoma: nodular sclerosis subtype was more prominent (55%) than mixed cellularity subtype (22.5%), which is similar to several European and US studies, lymphocyte rich (11.25%) and lymphocyte depleted (0%), while nodular lymphocyte predominant Hodgkin's lymphoma (11.25%).

CONCLUSIONS

Our study provided unique descriptive study of childhood HL, in Saudi Arabia, with valuable insight into the long-term outcome and late toxicity. Our results are comparable to other studies in the Middle East and European countries.

摘要

背景

霍奇金淋巴瘤(HL)是一种通常影响淋巴系统的淋巴肿瘤;它占沙特阿拉伯癌症的 3.6%。现代治疗方案在总生存率(OS)和无事件生存率(EFS)方面显示出了特别高的成功率。在我们的研究中,我们回顾了 2006 年 1 月至 2020 年 7 月在沙特阿拉伯利雅得的一家三级保健医院接受治疗的 80 名儿科和青少年 HL 患者的病历。探讨了人口统计学、临床和病理学数据。一线治疗方案为 ABVD、COG、COPP、R-CHOP 或单独放疗,分别用于 53/80(66.4%)、24/80(30%)、1/80(1.2%)、1/80(1.2%)或 1/80(1.2%)的患者;分别。在第一个 2 个周期后,通过 CT+/-PET 扫描进行第一次反应评估,然后每 2 个周期和治疗结束时进行评估。如果有任何复发的临床怀疑,也会进行另一次评估。

结果

中位年龄为 11 岁(范围 3-16 岁)。男女比例为 1.3:1。80 名患者中的 72 名(90%)表现出首次完全缓解(CR1),并保持缓解中位时间为 40 个月(范围 7-136 个月)。8 名患者出现难治性疾病。19 名患者接受挽救治疗(ICE 或 ESHAP/brentuximab vedotin 或 gemcitabine/brentuximab vedotin),14/19(73.7%)获得第二次完全缓解(CR2),中位时间为 24 个月(范围为 9-78 个月),而 5/19(26.3%)没有任何反应。5 年 OS 和 EFS 分别为 95%和 75%。两名患者患有 2 次恶性肿瘤,一名患有 AML 并死亡,另一名患有恶性纤维组织细胞瘤并仍存活。我们的患者均无生育问题。此外,他们没有经历慢性肺毒性或心脏毒性。经典霍奇金淋巴瘤:结节性硬化型(55%)比混合细胞型(22.5%)更为突出,这与欧洲和美国的几项研究相似,淋巴细胞丰富型(11.25%)和淋巴细胞耗竭型(0%),而结节性淋巴细胞为主型霍奇金淋巴瘤(11.25%)。

结论

我们的研究提供了沙特阿拉伯儿童 HL 的独特描述性研究,对长期结果和迟发性毒性有了宝贵的了解。我们的结果与中东和欧洲国家的其他研究相似。

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